scholarly journals A 54-Year-Old Woman with Progressive Ataxia

2016 ◽  
Vol 11 (3) ◽  
Author(s):  
Kun Huang MD PhD ◽  
Robin Parker MD FRCPC ◽  
Tabassum Firoz MD FRCPC

Paraneoplastic cerebellar degeneration (PCD) is a rare but devastating syndrome associated with autoantibodies targeting cerebellar antigens expressed by tumours outside of nervous system. PCD is most often associated with breast and gynecological cancers and can precede clinical manifestations of the cancer. Of the dozen autoantibodies associated with PCD, anti-Yo is the most commonly identified. In this report, we present a case of a woman with progressive cerebellar ataxia and anti-Yo antibody, who has microscopic high-grade serous carcinoma found with empiric total abdominal hysterectomy bilateral salpingo-oophorectomy (TAH-BSO). This case highlights the challenges in diagnosis, difficulty in identifying the occult malignancy, and the need for multidisciplinary collaboration between Internal Medicine, Neurology, Gynecology and Pathology. The literature relating to diagnosis, prognosis and treatment of PCD is also reviewed.

2020 ◽  
Vol 58 (231) ◽  
Author(s):  
Ramesh Dhakhwa ◽  
Anshu Vaidya ◽  
Amrita Giri ◽  
Archana Shakya ◽  
Achala Vaidya

A 49-year-old, perimenopausal nulliparous woman with lower abdominal pain and abnormal uterine bleeding. Clinical and radiological findings suggested a right adnexal tumor. CA-125 level was moderately elevated. Total abdominal hysterectomy with bilateral salpingo-oophorectomy was done. Peroperative findings revealed a soft to friable growth arising from right fallopian tube with no involvement of ovaries. Histopathologic examination confirmed it to be a high grade serous carcinoma, FIGO stage IA. The histomorphology resembled high grade serous carcinoma of ovary, however ovaries on both sides appeared unremarkable. Surgery was uneventful and the patient was discharged after seven days of hospital stay. She did not receive postoperative chemotherapy or radiotherapy and is under follow-up. The case is reported for its occurrence in an uncommon anatomic site and preoperative dilemma with relevant review of literature.


Hematology ◽  
2017 ◽  
Vol 2017 (1) ◽  
pp. 295-297 ◽  
Author(s):  
Patrick M. Reagan ◽  
Andrew Davies

Abstract A 60-year-old female presented with abdominal pain and distension. Following computed tomography scans of the abdomen and pelvis, she was taken urgently to the operating room, with the belief that she had appendicitis with perforation. At laparotomy, the findings were consistent with an ovarian carcinoma; there was extensive infiltration of the ovary, bowel, and omental deposits. Cytoreductive surgery was performed including total abdominal hysterectomy and bilateral salpingo-oophorectomy. The final pathology, however, revealed infiltration with medium-sized atypical lymphoid cells positive for CD20, CD10, MYC, BLC2, and BCL6 by immunohistochemistry. MYC and BCL2 translocations were identified by fluorescence in situ hybridization consistent with a diagnosis of high-grade B-cell lymphoma with rearrangements of MYC and BCL2. With the current data available, what is the optimal treatment of this patient?


2020 ◽  
Vol 3 (2) ◽  
pp. 392-394
Author(s):  
Alina Karna ◽  
Nisha Sharma

Adenoid basal carcinoma of the uterine cervix is a rare low-grade tumor and its cell origin is still obscure. Adenoid basal carcinoma can be confused with adenoid basal hyperplasia, adenoid cystic carcinoma, and basaloid squamous cell carcinoma of the cervix. We present here a case of a 59 year-old-female who initially presented with a high-grade squamous intraepithelial lesion on Pap smear. Total abdominal hysterectomy with bilateral salpingo-oophorectomy was performed. Histopathology revealed focal invasive adenoid basal carcinoma with extensive areas of a high-grade squamous intraepithelial lesion involving the endocervical gland. The immunohistochemical stain was positive for p16.


Rare Tumors ◽  
2016 ◽  
Vol 8 (4) ◽  
pp. 179-181 ◽  
Author(s):  
Yi Guo ◽  
Eleanor Chen ◽  
Darin J. Davidson ◽  
Venu G. Pillarisetty ◽  
Robin L. Jones ◽  
...  

Uterine leiomyosarcoma is a rare and aggressive malignancy with poor overall prognosis. There have been few reports of metastatic leiomyosarcoma in the gallbladder. We report a case of a 41-year-old female who underwent total abdominal hysterectomy due to presumed uterine fibroids. The postoperative pathology revealed high-grade pleomorphic leiomyosarcoma, with involvement of the uterine serosal surface. She subsequently underwent exploratory laparotomy, followed by pelvic radiation and chemotherapy. Since initial management she has developed metastatic disease and has been under treatment and surveillance for 11 years. She has undergone multiple surgical procedures and numerous lines of systemic therapy for metastatic leiomyosarcoma, including cholecystectomy for a metastatic lesion in the gallbladder. There have been no previous reports of metastatic leiomyosarcoma in the gallbladder. Despite extensive metastatic disease this patient has had prolonged survival with multi-modality management.


2021 ◽  
pp. 60-61
Author(s):  
JC Sharma ◽  
Anupma Anupma ◽  
Basanti Mazumdar ◽  
Dhruba Banik ◽  
Avir Sarkar

Undifferentiated endometrial sarcoma is a rare uterine malignancy of mesodermal origin. Only a few cases have been reported in literature. Herein, we describe a 56-year old woman who presented with post-menopausal bleeding of a short duration. Endometrial curettings were suggestive of undifferentiated sarcoma. Computed tomography showed an enlarged uterus with welldened mass in the endometrial cavity extending down to the cervix. A total abdominal hysterectomy, bilateral salpingo-oophorectomy, bilateral pelvic and para-aortic lymphadenectomy and omental biopsies were taken. Histological examination revealed a tumour with a permeative growth pattern composed of uniformly high grade round tumour cells with high mitotic activity. However, there was no lymphovascular space invasion. Tumour cells were strongly positive for CD10 signifying high grade endometrial stromal sarcoma (HG-ESS). Post R0 resection, patient is now receiving adjuvant chemotherapy. However, it is seen that most patients have early recurrence following even R0 resection.


2019 ◽  
Vol 12 (8) ◽  
pp. e229777 ◽  
Author(s):  
Eman Butt ◽  
John A Tadross ◽  
Karan R Chadda ◽  
John Latimer

This case describes a 69-year-old woman, who presented with rapidly progressive cerebellar symptoms and unintentional weight loss. Full neurological assessment excluded space-occupying lesions, vascular accidents and infection. Surprisingly, a chest, abdomen and pelvis CT showed a left hemipelvis mass, which was subsequently biopsied. A high-grade serous carcinoma of tubo-ovarian origin was found, diagnosing paraneoplastic cerebellar degeneration (PCD) secondary to this. The exact mechanism is not known, but is thought to be immune-mediated. In cases of PCD, after cancer treatment, the neurological disability stabilises to a severe level and will unfortunately be lifelong. Our patient continues to make great progress with intensive rehabilitation for her ongoing balance issues. Early recognition of PCD can lead to a prompt diagnosis of the underlying malignancy and hence subsequent management. This can at least limit the extent of the neurological disability of the disease and increase the survival rate from cancer.


2019 ◽  
Vol 152 (Supplement_1) ◽  
pp. S39-S39
Author(s):  
Francia Victoria De Los Reyes

Abstract Objectives Primary peritoneal serous carcinoma (PPSC) is defined as a high-grade malignant neoplasm with widespread peritoneal involvement in the setting of normal-sized ovaries that contain small surface tumoral implants and no other identifiable source of malignant serous neoplasm elsewhere in the gynecologic tract. Methods The aim of this case report is to contribute to the understanding of tumor immunophenotype and describe the behavior of a primary peritoneal serous carcinoma in relation to the conversion of the tumor’s WT1 immunohistochemistry expression from positive to negative after chemotherapy. Results This is a case of a 72-year-old female that presented with a history of increasing abdominal girth of 12 months. She underwent biopsy of the omentum and was diagnosed to have metastatic carcinoma that was positive for WT1, PAX8, CK7, and ER. The patient was initially diagnosed and managed as a case of ovarian serous carcinoma with metastasis to the omentum. She underwent neoadjuvant platinum-based chemotherapy and en-bloc total abdominal hysterectomy with bilateral salpingo-oophorectomy and low anterior resection, with metastasis resection. The criteria of PPSC were fulfilled due to the normal-sized ovaries and minimal ovarian involvement with normal fallopian tubes. Immunohistochemistry of the resection specimen showed negative WT1 and PAX8, and positive ER, CK, and p16 expression, with null p53 expression. Conclusion Following the concept that WT1 expression portends a poorer prognosis, the loss of WT1 expression may be a positive response to the chemotherapy and should be correlated with the time to recurrence. The patient was noted to be recurrence free for 1 year and still does not show signs of the disease at present. This is the first documented case of a previously WT1-positive primary peritoneal serous carcinoma that converted to a negative WT1 expression. This may provide insight on the role of chemotherapy in silencing protein expression.


2018 ◽  
Vol 2018 ◽  
pp. 1-5 ◽  
Author(s):  
Nuntasiri Eamudomkarn ◽  
Yuwadee Itarat ◽  
Pilaiwan Kleebkaow ◽  
Chumnan Kietpeerakool

High-grade endometrial stromal sarcoma (HG-ESS) is a rare clinical entity, particularly among young women, and only few cases have been reported in the literature. Herein, we describe the case of a 21-year-old woman who presented with a four-month history of excessive bleeding per vagina. Endometrial curettage and cervical biopsy revealed a malignant round cell tumor suggestive of metastatic sarcoma of uterine origin. Computed tomography of the abdominopelvic region showed an enlarged uterus with diffused thickening throughout the entire endometrial cavity. Intraabdominal lymphadenopathy and ascites in the pelvic cavity were noted. The patient underwent total abdominal hysterectomy, bilateral salpingo-oophorectomy, resections of the enlarged pelvic nodes, omentectomy, and biopsy of the peritoneal nodules in the cul-de-sac. Histological examination revealed a tumor with a permeative growth pattern composed of uniformly high-grade round cells with brisk mitotic activity and extensive lymphovascular space invasion. Sections of the pelvic lymph nodes on both sides and the peritoneal nodule revealed multiple metastatic foci. Immunohistochemical studies showed positive diffuse staining for vimentin, CD 10, and cyclin D1. The pathological diagnosis was HG-ESS stage IIIC. The patient experienced rapid progression of the disease while receiving adjuvant treatment and succumbed eight months after the operation. HG-ESS is a rare cause of AUB in adolescents and young women but should be considered in the differential diagnosis.


Author(s):  
B Archana ◽  
Sandhya Sundaram ◽  
Lawrence D Cruze ◽  
KS Rajeshwari

Uterine sarcomas are relatively rare and account for only 1-3% of all malignancies in the Female Genital Tract (FGT). Adenosarcomas of the uterus are uncommon accounting for only 5% of uterine sarcomas. They are considered less recurrent and less metastatic. However, those with sarcomatous overgrowth and heterologous differentiation are very rare, considered aggressive and need to be differentiated histologically from other sarcomas that mimic the same clinically and histologically. In the index case, a 50-year-old female presented with symptoms of pain in the abdomen and bleeding per vagina. Magnetic Resonance Imaging (MRI) showed a large polypoid solid mass in the endometrium, suggestive of a sarcoma. Positron Emission Tomography (PET) scan showed an Fluorodeoxyglucose (FDG) avid lesion in the endometrial cavity (SUV max-13.33). Total abdominal hysterectomy and salpingooophorectomy was performed. Histology showed a biphasic tumour (benign glands and malignant mesenchymal component) with rhabdomyoblastic differentiation and sarcomatous overgrowth. Presence of rhabdoid cells raises the possibility of other sarcomas with these features making the diagnosis challenging. Immunohistochemistry (IHC) confirmed it to be a high grade adenosarcoma with sarcomatous overgrowth. Desmin positivity was noted in the rhabdoid cells. Majority cases of adenosarcoma are low grade, the index case was however high grade with presence of heterologous elements and sarcomatous overgrowth. Neither radiology nor clinical features can reliably differentiate adenosarcomas from other uterine sarcomas. A definitive diagnosis can be made by understanding the overlapping morphological features and correlate it with IHC to reliably differentiate it with other similar looking uterine sarcomas.


2020 ◽  
Vol 7 (1) ◽  
pp. 360-363
Author(s):  
Hacı Öztürk Şahin ◽  
Mehmet Bayrak

Objective: Endometrial stromal sarcoma (ESS) is a rare mesenchymal tumor of the uterus.Literature has limited data about the ESS. The aim of the present study was to contribute to literature by reporting the histo-pathological findings, clinical characteristics of ESS patients and the data about the accuracy of preoperative diagnosis and prognosis. Material and Methods: A total of 33 patients who were diagnosed and followed up with ESS at Department of Gynecology and Obstetrics of Bursa Uludağ University between 2007 and 2017 were retrospectively analyzed with regard to clinical and pathologic characteristics, surgical procedures they underwent and survival. Results: Mean age of the patients was 49.5 years and 60.2 years for low grade ESS (LG-ESS) and high grade ESS (HG-ESS) (p=0,01). Post-menopausal hemorrhage was the most common complaint on admission.  Correct histological diagnosis was made in only 72.7% of the patients from whom pre-operative endometrial biopsy was obtained. Twelve out of 16 cases (75%) were in Stage 1. While all patients underwent total abdominal hysterectomy and bilateral salpingo-oophorectomy (TAH+BSO), 14 underwent pelvic and para-aortic lymphadenectomy for surgical staging. Lymph node involvement was detected in no patients who underwent lymphadenectomy. The patients with LG-ESS were found to have a good prognosis however the ones with HG-ESS had a high mortality rate even in the early stages (mean survival of 10 months). Conclusion: High grade ESS cases show different clinical characteristics and prognosis than LG-HSS. Diagnostic accuracy of endometrial sampling is much lower when compared to epithelial uterine malignancies. Metastasis of pelvic-paraaortic lymph nodes of which removal is reported which not to contribute to survival is rare.


Sign in / Sign up

Export Citation Format

Share Document